Venooclusive disease

Hepatic veno-occlusive disease/sinusoidal obstruction syndrome (VOD/SOS) is one of the most life-threatening early complications following hematopoietic cell transplantation (HCT). Due to the high mortality rate of severe VOD/SOS accompanied with multiorgan failure, there is a great interest in preventive strategies. The efficacy of defibrotide (DF) on the prevention of VOD/SOS has been clearly shown in high-risk pediatric patients, but evidence-based data on adults is scarce. In this report, we aimed to assess the impact of DF on the incidence of VOD/SOS in our center by posttransplant day 30 among patients who were treated with allogeneic HCT (allo-HCT)...

BACKGROUND: Erectile dysfunction (ED) is the second sexual dysfunction affecting men. Penile duplex ultrasound (PDU) with intracavernous injection of a vasoactive agent as alprostadil or papaverine, may play an important role in differentiating psychogenic from vasculogenic ED (arterial or venooclusive) and may also have an important role in the secondary prevention of cardiovascular events. The aim of this study is to investigate the relationship between the vascular parameters and sexual satisfaction as established by a questionnaire...

We prospectively analyzed outcomes of haploidentical hematopoietic stem cell transplantation using αβ+ T-cell receptor/CD19+ depleted grafts. Sixty-three transplantations were performed in 60 patients. Twenty-eight patients were diagnosed with acute lymphoblastic leukemia (ALL), 27 patients were diagnosed with acute myelogenous leukemia, and in eight other hematological malignancies were diagnosed. Twenty-three were in first complete remission (CR), 20 in second CR, 20 beyond second CR. Four patients developed graft failure...

Budd-Chiari syndrome (BCS) is a rare disease with an incidence of 0.1 to 10 per million inhabitants a year caused by impaired venous outflow from the liver mostly at the level of hepatic veins and inferior vena cava. Etiological factors include hypercoagulable conditions, myeloprolipherative diseases, anatomical variability of the inferior vena cava, and environmental conditions. Survival rates in treated patients range from 42 to 100% depending on the etiology and the presence of risk factors including parameters of Child-Pugh score, sodium and creatinine plasma levels, and the choice of treatment...

INTRODUCTION AND OBJECTIVES: Pulmonary arterial hypertension (PAH) is characterized by increased pulmonary vascular resistance, right ventricular dysfunction and death. Despite scientific advances, is still associated with high morbidity and mortality. The aim is to describe the clinical approach and determine the prognostic factors of patients with PAH treated in a national reference center over 30 years. METHODS: Three hundred and seventy nine consecutive patients with PAH (January 1984 to December 2014) were studied...

Cytomegalovirus (CMV) retinitis remains the most common opportunistic ocular infection in immunocompromised patients. Patients with immunocompromising diseases, such as acquired immunodeficiency syndrome, inherited immunodeficiency states, malignancies, and those on systemic immunosuppressive therapy, are known to be at risk. Recently, it has been suggested that patients undergoing intravitreal injection of immunosuppressive agents may also be predisposed. One previous case report speculated that there may be an additional risk for CMV retinitis in acquired immunodeficiency syndrome patients with venoocclusive disease...

No reliable therapy has yet been established for unresectable hepatocellular carcinoma (HCC). Encouraging data in terms of response rate and survival have been reported with intra-arterial chemotherapy combined with venooclusive materials, specifically ethiodized oil and gelatin sponge. To evaluate the activity and tolerance of a new chemoembolization protocol in cirrhotic patients with HCC, 22 patients were treated with epirubicin (50 mg) and ethiodized oil (10-15 ml), administered through hepatic arterial catheters, followed by gelatin sponge...

Priapism is defined as persistent erection without sexual stimulus. The new classifications make a distinction between venooclusive low flow priapism, isquemic and arterial high flow priapism, non isquemic. The perineal or penis trauma are responsible the most of cases arterial priapism, formation of an arteriocavernous fistula. The diagnosis is clinical, blood gas analysis and colour Doppler ultrasonography of the corpora cavernosa confirmed the diagnosis. The arteriography with selective embolization constitutes a safe and effective therapeutic method...

A 19-year-old woman was treated for recurrent sinusitis with oral trovafloxacin and developed acute hepatitis and peripheral eosinophilia, with hepatosplenomegaly and ascites. Laparoscopic liver biopsy showed extensive centrilobular hepatocyte necrosis, likely causing venooclusive disease-like signs and symptoms. Clinical and laboratory abnormalities resolved completely after prolonged treatment with steroids. The temporal relationship between trovafloxacin and the onset of hepatitis favors this drug as a culprit...

We report the novel use of the transjugular intrahepatic portosystemic shunt (TIPS) procedure for the treatment of intractable ascites due to hepatic venooclusive disease as a result of whole abdominopelvic radiotherapy. A patient with Stage III endometrioid carcinoma of the endometrium treated with postoperative whole abdominopelvic irradiation developed intractable ascites. Multiple paracenteses and computerized tomography were negative for recurrent carcinoma. Liver biopsy demonstrated hepatic venoocclusive disease, a rare complication of therapeutic radiation involving the liver...

Pulmonary arterial hypertension has many causes, only some of which are well understood. The radiographic findings in pulmonary arterial hypertension are very similar regardless of the cause. Some radiographic features such as cardiac chamber enlargement, occur secondary to elevation of pulmonary pressures. In addition, sequential radiographs may demonstrate dramatic changes as the hypertension develops. This article presents examples of the various forms of pulmonary arterial hypertension, with pathologic correlation...

Forty-two cases with Wilms' tumor encountered in the National Taiwan University Hospital from 1978 through 1989 were retrospectively reviewed. Included were 19 boys and 23 girls, with an age range at diagnosis from 7 days to 10 years; a majority were in the first 6 years of life. The presenting symptoms and signs included: abdominal mass (89.2%), hypertension (57.9%), hematuria (28.2%), gastrointestinal symptoms (26.3%), fever (24.3%), and body weight loss (21.6%). The initial laterality of tumor was 28 right and 14 left, with one contralateral and one ipsilateral relapse...

RhGM-CSF is a hematopoietic growth factor which stimulates the proliferation, differentiation and functional activity of neutrophils, monocytes and macrophages. It also stimulates proliferation of endothelial cells and induces the production of other cytokines, such as interleukin (IL-1), tumor necrosis factor (TNF), interferon, prostaglandin E2, and plasminogen activating factor which affects both hematopoietic and non-hematopoietic cell activities. Initial clinical studies in 1987 generally excluded experimental therapy with rhGM-CSF in pediatric patients (age < or = 17 years) unless life threatening illness related to neutropenia and infection developed (i...